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Clinical Management of Pyoderma Gangrenosum

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Abstract

Pyoderma gangrenosum is a noninfectious neutrophilic dermatosis that usually starts with sterile pustules which rapidly progress to painful ulcers of variable depth and size with undermined violaceous borders. In 17 to 74% of cases, pyoderma gangrenosum is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatological or hematological disease or malignancy. Diagnosis of pyoderma gangrenosum is based on a history of an underlying disease, typical clinical presentation and histopathology, and exclusion of other diseases that would lead to a similar appearance.

Randomized, double-blinded prospective multicenter trials investigating the treatment of pyoderma gangrenosum are not available. The treatments with the best clinical evidence are systemic corticosteroids (in the initial phase usually 100 to 200 mg/day) and cyclosporine (mainly as a maintenance treatment). Combinations of corticosteroids with cytotoxic drugs such as azathioprine, cyclophosphamide or chlorambucil are used in patients with disease that is resistant to corticosteroids. The combination of corticosteroids with sulfa drugs, such as dapsone, or clofazimine, minocycline and thalidomide, has been used as a corticosteroid-sparing alternative. Limited experience has been documented with methotrexate, colchicine, nicotine, and mycophenolate mofetil, among other drugs. Alternative treatments include local application of granulocyte-macrophage colony-stimulating factor, intravenous immunoglobulins and plasmapheresis. Skin transplants (split-skin grafts or autologous keratinocyte grafts) and the application of bioengineered skin is useful in selected cases in conjunction with immunosuppression. Topical therapy with modern wound dressings is useful to minimize pain and the high risk of secondary infection. The application of topical antibacterials cannot be recommended because of their potential to sensitize and their questionable efficacy, but systemic antibacterial therapy is mandatory when infection is present. Despite recent advances in therapy, the prognosis of pyoderma gangrenosum remains unpredictable.

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References

  1. Brunsting L.A., Goeckerman W.H., O’Leary P.A. Pyoderma (ecthyma) gangrenosum: clinical and experimental observations in five cases occuring in adults. Arch Dermatol Syph 1930; 22: 655–80

    Article  Google Scholar 

  2. Kuner N., Hartschuh W. Darstellung des Pyoderma gangraenosum in einem dermatologischen atlas des frühen 19: Jahrhunderts. Hautarzt 2000; 51: 519–23

    Article  PubMed  CAS  Google Scholar 

  3. Perry H.O. Pyoderma gangrenosum. South Med J 1969; 62: 899–908

    Article  PubMed  CAS  Google Scholar 

  4. Van den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol 1997; 137: 1000–5

    Article  PubMed  Google Scholar 

  5. Graham J.A., Hansen K.K., Rabinowitz L.G., et al. Pyoderma gangrenosum in infants and children. Pediatr Dermatol 1994; 11: 10–7

    Article  PubMed  CAS  Google Scholar 

  6. Dick D.C., Mackie R.M., Patrick W.J.A., et al. Pyoderma gangrenosum in infancy. Acta Derm Venereol 1982; 62: 348–50

    PubMed  CAS  Google Scholar 

  7. Fulbright R.K., Wolf J.E., Tschen J.A. Pyoderma gangrenosum at surgery sites. J Dermatol Surg Oncol 1985; 11: 883–6

    PubMed  CAS  Google Scholar 

  8. Su W.P.D., Schroeter A.L., Perry H.O., et al. Histopathologic and immunopathologic study of pyoderma gangrenosum. J Cutan Pathol 1986; 13: 323–30

    Article  PubMed  CAS  Google Scholar 

  9. Shaya S., Kindzelskii A.L., Minor J., et al. Aberrant integrin (CR4; αxß2; CD11c/CD18) oscillation on neutrophils in a mild form of pyoderma gangrenosum. J Invest Dermatol 1998; 111: 154–8

    Article  PubMed  CAS  Google Scholar 

  10. Adachi Y., Kindzelskii A.L., Cookingham G., et al. Aberrant neutrophil trafficking and metabolic oscillations in severe pyoderma gangrenosum. J Invest Dermatol 1998; 111: 259–68

    Article  PubMed  CAS  Google Scholar 

  11. Tanaka N., Fujioka A., Tajima S., et al. Elafin is induced in epidermis in skin disorders with dermal neutrophilic infiltration: interleukin-1 beta and tumour necrosis factor-alpha stimulate ist secretion in vitro. Br J Dermatol 2000; 143: 728–32

    Article  PubMed  CAS  Google Scholar 

  12. Wines M.E., Lee L., Katari M.S., et al. Identification of mesoderm development (mesd) candidate genes: comparative mapping and genome sequence analysis. Genomics 2001; 72: 88–98

    Article  PubMed  CAS  Google Scholar 

  13. Schwaegerle S.M., Bergfeld W.F., Senitzer D., et al. Pyoderma gangrenosum: a review. J Am Acad Dermatol 1988; 18: 559–68

    Article  PubMed  CAS  Google Scholar 

  14. Van Hale H.M., Rogers R.S., Zone J.J., et al. Pyostomatitis vegetans: a reactive mucosal marker for inflammatory disease of the gut. Arch Dermatol 1985; 1215: 94–8

    Google Scholar 

  15. Krüger S., Piroth W., Amo Takyi B., et al. Multiple aseptic pulmonary nodules with central necrosis in a patient with pyoderma gangrenosum. Chest 2001; 119: 977–8

    Article  PubMed  Google Scholar 

  16. Marie I., Levesque H., Joly P., et al. Neutrophilic myositis as an extracutaneous manifestation of neutrophilic dermatosis. J Am Acad Dermatol 2001; 44: 137–9

    Article  PubMed  CAS  Google Scholar 

  17. Prystowsky J.H., Kahn S.N., Lazarus G.S. Present status of pyoderma gangrenosum. Arch Dermatol 1989; 125: 57–64

    Article  PubMed  CAS  Google Scholar 

  18. Powell F.C., Su W.R.D., Perry H.O. Pyoderma gangrenosum: classification and management. J Am Acad Dermatol 1996; 34: 395–409

    Article  PubMed  CAS  Google Scholar 

  19. Hurwitz R.M., Haseman J.H. The evolution of pyoderma gangrenosum: a clinicopathologic correlation. Am J Dermatopathol 1993; 15: 28–33

    Article  PubMed  CAS  Google Scholar 

  20. Winkelmann R.K., Wilson-Jones E., Gibson L.E., et al. Histopathologic features of superficial granulomatous pyoderma gangrenosum. J Dermatol 1989; 16: 127–32

    PubMed  CAS  Google Scholar 

  21. Park H.J., Kim Y.C., Cinn Y.W., et al. Granulomatous pyoderma gangrenosum: two unusual cases showing necrotizing granulomatous inflammation. Clin Exp Dermatol 2000; 25: 617–20

    Article  PubMed  CAS  Google Scholar 

  22. Bennett M.L., Jackson J.M., Jorizzo J.L., et al. Pyoderma gangrenosum: a comparison of typical and atypical cases with an emphasis on time to remission, case review of 86 patients of 2 institutions. Medicine (Baltimore) 2000; 79: 37–46

    Article  CAS  Google Scholar 

  23. Burton J.L. Sweet’s syndrome: pyoderma gangrenosum and acute leukaemia [letter]. Br J Dermatol 1980; 102: 239

    Article  PubMed  CAS  Google Scholar 

  24. Bernstein C.N., Blanchard J.F., Rawsthorne P., et al. The prevalence of extraintestinal diseases in inflammatory bowel disease: a population-based study. Am J Gastroenterol 2001; 96: 1116–22

    Article  PubMed  CAS  Google Scholar 

  25. Cooper P. TenderWet: an innovation in moist wound healing. Br J Nurs 1998; 7: 1232–5

    PubMed  CAS  Google Scholar 

  26. Auböck J. Synthetic dressings. Curr Probl Dermatol 1999; 27: 26–48

    Article  PubMed  Google Scholar 

  27. Wollina U. Moderne Wunddressings: ein Update. Vasomed 1997; 9 Suppl.: 148–52

    Google Scholar 

  28. Wollina U., Liebold K., Konrad H. Topical treatment of malignant wounds. Eur J Geriatrics 2001; 3: 118–21

    Google Scholar 

  29. Kramer A., Adrian V., Rudolph P., et al. Explanttest mit Haut und Peritoneum der neonatalen Ratte als prädiktiver Toleranztest für lokale anti-infektiöse Substanzen für Wunden und Körperkavitäten. Chirurg 1998; 69: 840–5

    Article  PubMed  CAS  Google Scholar 

  30. Wollina U. Contact allergy associated with topical treatment. In: Gebhardt M., Elsner P., Marks Jr J.G., editors. Handbook of contact dermatitis. London: Martin Dunitz Publishers, 2000: 71–81

    Google Scholar 

  31. Ortonne J.P. A controlled study of the activity of hyaluronic acid in the treatment of venous leg ulcers. J Dermatol Treat 1996; 7: 75–81

    Article  Google Scholar 

  32. Wollina U., Karamfilov T. Treatment of recalcitrant ulcers in pyoderma gangrenosum with mycophenolate mofetil and autologous keratinocyte transplantation on a hyaluronic acid matrix. J Eur Acad Dermatol Venereol 2000; 14: 187–90

    Article  PubMed  CAS  Google Scholar 

  33. Goldstein F., Krain R., Thornton J.J. Intralesional steroid therapy of pyoderma gangrenosum. J Clin Gastroenterol 1985; 7: 499–501

    Article  PubMed  CAS  Google Scholar 

  34. Abu-Elmagd K., Van Thiel D.H., Jegasothy B.V., et al. Resolution of severe pyoderma gangrenosum in a patient with streaking leukocyte factor disease after treatment with tacrolimus (FK-506). Ann Intern Med 1993; 119: 595–8

    PubMed  CAS  Google Scholar 

  35. Reich K., Vente C., Neumann C. Topical tacrolimus for pyoderma gangrenosum. Br J Dermatol 1998; 139: 755–7

    Article  PubMed  CAS  Google Scholar 

  36. Petering H., Kiehl P., Kapp A., et al. Pyoderma gangraenosum: erfolgreiche topische Therapie mit Tacrolimus (FK506). Hautarzt 2001; 52: 47–50

    Article  PubMed  CAS  Google Scholar 

  37. Lyon C.C., Smith A.J., Beck M.H., et al. Parastomal pyoderma gangrenosum: clinical features and management. J Am Acad Dermatol 2000; 42: 992–1002

    Article  PubMed  CAS  Google Scholar 

  38. Theissen U., Luger T.A., Schwarz T. Erfolgreiche topische Anwendung von Cyclosporin A bei Pyoderma gangraenosum. Hautarzt 1996; 47: 132–5

    Article  PubMed  CAS  Google Scholar 

  39. Mrowietz U., Christophers E. Clearing of pyoderma gangrenosum by intralesional cyclosporin A [letter]. Br J Dermatol 1991; 125: 499

    Article  PubMed  CAS  Google Scholar 

  40. Langenbach N., Goetz A., Hohenleutner U., et al. Effectiveness of 4% disodium chromoglycate in the treatment of pyoderma gangrenosum. Acta Derm Venereol 1996; 76: 501–2

    PubMed  CAS  Google Scholar 

  41. Anderson L.L., Samlaska C.P., Cardone J.S., et al. Treatment of pyoderma gangrenosum with 4% cromolyn. Arch Dermatol 1994; 130: 1117–20

    Article  PubMed  CAS  Google Scholar 

  42. Wolf R., Wolf D., Ruocco V. The benefits of smoking in skin disease. Clin Dermatol 1998; 16: 641–7

    Article  PubMed  CAS  Google Scholar 

  43. Kanekura T., Kanzaki T. Successful treatment of pyoderma gangrenosum with nicotine chewing gum. J Dermatol 1995; 22: 704–5

    PubMed  CAS  Google Scholar 

  44. Groves R.W., Schmidt-Lucke J.A. Recombinant human GM-CSF in the treatment of poorly healing wounds. Adv Skin Wound Care 2000; 13: 107–12

    PubMed  CAS  Google Scholar 

  45. Ross H.J., Moy L.A., Kaplan R., et al. Bullous pyoderma gangrenosum after granulocyte colony-stimulating factor treatment. Cancer 1991; 68: 441–3

    Article  PubMed  CAS  Google Scholar 

  46. Lyon C., Stapleton M., Smith A., et al. Topical sucralfate in the management of peristomal skin disease: an open study. Clin Exp Dermatol 2000; 25: 584–8

    Article  PubMed  CAS  Google Scholar 

  47. Johnson R.B., Lazarus G.S. Pulse therapy: therapeutic efficacy in the treatment of pyoderma gangrenosum. Arch Dermatol 1982; 118: 76–84

    Article  PubMed  CAS  Google Scholar 

  48. Matis W.L., Ellis C.N., Griffiths C.E.M., et al. Treatment of pyoderma gangrenosum with cyclosporine. Arch Dermatol 1992; 128: 1060–4

    Article  PubMed  CAS  Google Scholar 

  49. O’Donnell B., Powell F.C. Cyclosporin treatment of pyoderma gangrenosum. J Am Acad Dermatol 1991; 24: 141–3

    Article  PubMed  Google Scholar 

  50. Zumdick M., Goerz G., Schuppe H.C., et al. Niedrig dosierte Cyclosporin-A-Therapie bei Pyoderma gangraenosum: erfahrungen bei 6 Patienten. Hautarzt 1995; 46: 697–701

    Article  PubMed  CAS  Google Scholar 

  51. Callen J.P. Pyoderma gangrenosum and related disorders. Adv Dermatol 1989; 4: 51–70

    PubMed  CAS  Google Scholar 

  52. Arbiser J.L., Moschella S.L. Clofazimine: a review of its medical uses and mechanisms of action. J Am Acad Dermatol 1995; 32: 241–7

    Article  PubMed  CAS  Google Scholar 

  53. Peuckmann V., Fisch M., Bruera E. Potential novel uses of thalidomide: focus on palliative care. Drugs 2000; 60: 273–92

    Article  PubMed  CAS  Google Scholar 

  54. Michel S., Hohenleutner U., Mohr V., et al. Therapieresistentes Pyoderma gangraenosum: eine Behandlung mit Mycophenolatmofetil und CyclosporinA. Hautarzt 1999; 50: 438–41

    Google Scholar 

  55. Teitel A.D. Treatment of pyoderma gangrenosum with methotrexate. Cutis 1996; 57: 326–8

    PubMed  CAS  Google Scholar 

  56. Newell L.M., Malkinson F.D. Pyoderma gangrenosum: response to cyclophosphamide therapy. Arch Dermatol 1985; 113: 601–4

    Google Scholar 

  57. Burruss J.B., Farmer E.R., Callen J.P. Chlorambucil is an effective corticosteroidsparing agent for recalcitrant pyoderma gangrenosum. J Am Acad Dermatol 1996; 35: 720–4

    Article  PubMed  CAS  Google Scholar 

  58. Wollina U., Looks A., Kammler H.J. Intravenous immunoglobulin therapy in dermatology; review and center experience. Anais Bras Dermatol (Rio de Janeiro) 1998; 73: 255–9

    Google Scholar 

  59. Gupta A.K., Shear N.H., Sauder D.N. Efficacy of human immune globulin in pyoderma gangrenosum. J Am Acad Dermatol 1995; 32: 140–2

    Article  PubMed  CAS  Google Scholar 

  60. Dirschka T., Kastner U., Behrens S., et al. Successful treatment of pyoderma gangrenosum with intravenous human immunoglobulin. J Am Acad Dermatol 1998; 39: 789–90

    Article  PubMed  CAS  Google Scholar 

  61. Smith J.B., Shenefelt P.D., Soto O., et al. Pyoderma gangrenosum in a patient with cryoglobulinemia and hepatitis C successfully treated with interferon alfa. J Am Acad Dermatol 1996; 34: 901–3

    Article  PubMed  CAS  Google Scholar 

  62. Kaminska R., Ikaheimo R., Hollmen A. Plasmapheresis and cyclophosphamide as successful treatments for pyoderma gangrenosum. Clin Exp Dermatol 1999; 24: 81–5

    Article  PubMed  CAS  Google Scholar 

  63. Paolini O., Hebuterne X., Flory P., et al. Treatment of pyoderma gangrenosum with colchicine. Lancet 1995; 345: 1057–8

    Article  PubMed  CAS  Google Scholar 

  64. Wasserteh V., Bruce S., Sessoms S.L., et al. Pyoderma gangrenosum treated with hyperbaric oxygen therapy. Int J Dermatol 1992; 31: 594–6

    Article  Google Scholar 

  65. Tan M.H., Gordon M., Lebwohl O., et al. Improvement of pyoderma gangrenosum and psoriasis associated with Crohn disease with anti-tumor necrosis factor alpha monoclonal antibody. Arch Dermatol 2001; 137: 930–3

    PubMed  CAS  Google Scholar 

  66. MacKenzie D., Moiemen N., Frame J.D. Pyoderma gangrenosum following breast reconstruction. Br J Plast Surg 2000; 53: 441–3

    Article  PubMed  CAS  Google Scholar 

  67. Castro-Duran J., Martin-Armada M., Jimenez-Alonso J. Pyoderma gangrenosum induced by acupuncture in a patient with ulcerative colitis [letter]. Arch Intern Med 2000; 160: 2394

    Article  PubMed  CAS  Google Scholar 

  68. Havlik R.J., Giles P.D., Havlik N.L. Pyoderma gangrenosum of the breast: sequential grafting. Plast Reconstr Surg 1998; 101: 1909–14

    Article  PubMed  CAS  Google Scholar 

  69. Cliff S., Holden C.A., Thomas P.R., et al. Split skin grafts in the treatment of pyoderma gangrenosum: a report of four cases. Dermatol Surg 1999; 25: 299–302

    Article  PubMed  CAS  Google Scholar 

  70. Hafner J., Trüeb R.M. Management of vasculitic leg ulcers and pyoderma gangrenosum. Curr Probl Dermatol 1999; 27: 277–85

    Article  PubMed  CAS  Google Scholar 

  71. Alam M., Grossman M.E., Schneiderman P.I., et al. Surgical management of pyoderma gangrenosum: case report and review. Dermatol Surg 2000; 26: 1063–6

    Article  PubMed  CAS  Google Scholar 

  72. Gallico G.G. Biologic skin substitutes. Clin Plast Surg 1990; 17: 519–26

    PubMed  Google Scholar 

  73. Hunyadi J., Farkas B., Olah J., et al. Keratinocyte grafting; a new means of transplantation for full thickness wounds. J Dermatol Surg Oncol 1988; 14: 75–8

    PubMed  CAS  Google Scholar 

  74. Limat A., Mauri D., Hunziker T. Successful treatment of chronic leg ulcers with epidermal equivalents generated from cultured autologous outer root sheath cells. J Invest Dermatol 1996; 101: 128–35

    Article  Google Scholar 

  75. Dean S.J., Nieber S., Hickerson W.L. The use of cultured epithelial autograft in a patient with idiopathic pyoderma gangrenosum. Ann Plast Surg 1991; 26: 194–5

    Article  PubMed  CAS  Google Scholar 

  76. Limova M., Mauro T. Treatment of pyoderma gangrenosum with cultured keratinocyte autografts. J Dermatol Surg Oncol 1994; 20: 833–6

    PubMed  CAS  Google Scholar 

  77. De Imus G., Golomb C., Wilkel C., et al. Accelerated healing of pyoderma gangrenosum treated with bioengineered skin and concomitant immunosuppression. J Am Acad Dermatol 2001; 44: 61–6

    Article  PubMed  Google Scholar 

  78. Rozen S.M., Nahabedian M.Y., Manson P.N. Management strategies for pyoderma gangrenosum: case studies and review of the literature. Ann Plast Surg 2001; 47: 310–5

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

The author gratefully acknowledges the cooperation of Helga Konrad, Dr Theodor Karamfilov and Dr Kristin Liebold.

This paper is dedicated to Professor Claus Seebacher, former director of the Department of Dermatology at Dresden-Friedrichstadt.

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  1. Department of Dermatology, Hospital Dresden-Friedrichstadt, PO Box 120906, Dresden, 01008, Germany

    Uwe Wollina

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Wollina, U. Clinical Management of Pyoderma Gangrenosum. Am J Clin Dermatol 3, 149–158 (2002). https://doi.org/10.2165/00128071-200203030-00002

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